Unprotected People Reports: Chickenpox

The Immunization Action Coalition (IAC) publishes stories
of people who have suffered or died from
vaccine-preventable diseases and occasionally devotes an IAC
Express issue to such a story. This is the
43rd story in our series. We usually try to choose
stories that are written for a general audience.
Sometimes, however, a powerful story
appears in a technical medical journal that we
think almost all readers can appreciate. We
believe that today's story about an adult case of
varicella infection is
indeed powerful. For those who don't have a
medical dictionary handy, "orbital exenteration" means
complete removal of the contents of the eye socket.

Most of us think of chickenpox, or varicella infection, as
a childhood disease. It's true that many more
children than adults get chickenpox.
However, a higher proportion of adults than
children die from chickenpox or complications
from the disease. The following medical case report tells us
about one adult male, previously healthy,
who apparently caught chickenpox from his infected
children. He died after a
year of suffering from pneumonia and severe group A
Streptococcus infection that led to
varicella gangrenosa, a form of necrotizing
fasciitis.

Adults, if you have not had chickenpox in the past, it's not
too late to get vaccinated now. Ask your
doctor.

"Periorbital Varicella Gangrenosa Necessitating Orbital
Exenteration in a Previously Healthy Adult" was
written by William O. Thomas, M.D.; James
A. Parker, M.D.; Bonnie Weston, M.D.; and
Christine Evankovich, M.D. The full
text, excluding two figures, is republished here by
permission of the Southern Medical
Association, which originally published this
article in July 1996 in the Southern Medical
Journal. (Copyright Southern Medical
Association.)

AbstractA previously healthy 31-year-old man had profound
neurologic compromise and necrotizing periorbital
infection due to a complication of varicella infection.
Despite aggressive treatment, he required orbital
exenteration and radical debridement of the involved
tissues. He survived in a vegetative state for
almost 1 year before succumbing to
progressive neurologic deterioration. We present
and discuss this complicated
case of varicella gangrenosa to show the devastating
nature of complications that can occur from
varicella infection in the unsensitized adult.

Although varicella is generally considered a benign
disease of childhood, complications can occur.
Our report of devastating complications
from varicella infection in a previously
unsensitized adult is presented to alert the
readership to the potential for development of varicella
gangrenosa in adults who have chickenpox.

Case ReportA 31-year-old white man was seen initially at an outlying
hospital, where a diagnosis of chickenpox was
made. His children had recently been
infected with chickenpox, and he had no antecedent
history of having had the disease as
a child. Previously, he had been otherwise healthy.
Despite conservative measures, he continued
to have high fevers and went to a local emergency
room with evidence of dehydration and septicemia.
While he was in the emergency room, neurologic
deterioration and coma developed. The
patient was transferred to the University of South Alabama
and was admitted to the medical intensive
care unit, where mechanical ventilation was begun
because of pulmonary insufficiency and respiratory
arrest due to sepsis and airway swelling. He had
hypotension with clinical signs of
septicemia, shock, renal failure, and encephalitis and was
treated with intravenous acyclovir (800 mg
every 12 hours) and appropriate dosages of
nafcillin, ceftazidime (Fortaz), and metronidazole
for broad spectrum antibiotic coverage
because of the renal compromise. Evaluation in our
institution showed necrotic eyelids, as well as
significant surrounding facial and neck erythema and
cellulitis.

Blood cultures and vesicular cultures were positive for
group A [beta]-hemolytic Streptococcus sp.
Computed tomography of the neck, face, and brain
showed significant diffuse brain swelling and
cerebritis with impending herniation.
Neurosurgical consultation was obtained. High-dose steroid
therapy was recommended and instituted to
prevent herniation from the brain swelling and edema. The
plastic surgery service saw this patient in consultation
with the ophthalmology service for evaluation of
the periorbital swelling and lid necrosis,
and immediate operative debridement was scheduled.

After neurosurgical clearance, the patient was brought to
the operating theater for debridement of the
necrotic lids and decompression of the
tense fluid accumulation in the neck. At the time
of debridement of the necrotic lids, we
found subcutaneous fat necrosis with some purulence
throughout all planes of the incised left
side of the face. Frozen section examination of
debridement specimens was negative
for mucormycosis. Additionally, there was
some necrotic bone of the left zygoma, which was also
debrided. The left side of the neck was incised, yielding
about 500 mL of serosanguineous fluid from
the left and 300 mL of fluid from the right side.
The left globe appeared marginally viable but was
left in situ at the
initial debridement, in hopes that the retinal arterial
blood supply would allow preservation of
left eye sight. After the left globe was observed
for a few more days, it became obviously necrotic.
Orbital exenteration was recommended and
done along with further debridement of the
periorbital structures, which had developed
further gangrenous changes despite our initially
debriding back to bleeding apparently viable
tissues, including periorbital bone. Wound
cultures were positive also for group A
[beta]-hemolytic Streptococcus sp.

The patient continued to fare poorly with signs and
symptoms of continued sepsis and was maintained
by vigorous supportive measures including
tube feeding, mechanical ventilation, a pressure
prevention bed, and
wet-to-dry dressing changes for the orbital exenteration
defect. A left frontal lobe brain abscess
developed and was drained by stereotactic
craniotomy. The neurologic status did not improve,
and the patient remained in a vegetative
state with essentially no organized cortical brain
wave activity on serial electroencephalograms.
Pulmonary support was difficult but aided by tracheostomy
for pulmonary toilet, and with frequent
suctioning he survived.

Intermittent pneumonia and atelectasis of both lungs with
low systemic arterial oxygen saturations
precluded a safe anesthetic for the performance of
additional elective operations. Approximately 3
months after hospital admission, he was
transferred to a chronic care and rehabilitation
facility with the left periorbital wound
manifesting continued contraction and slow wound healing.
The periorbital wound epithelialized
eventually without requiring skin grafting. He
survived in a vegetative state at the
rehabilitation facility with no sign of any higher
cortical function and died approximately 1 year after the
onset of his illness.

DiscussionVaricella is generally considered a benign disease in
childhood. In a 1935 study of hospitalized
children, a 5.2% complication rate was reported
among more than 2,500 patients with chickenpox.[1]
In ambulatory patients, the complication
rate must be significantly lower. In adults, the
complication rate of varicella infection is severalfold higher, and results of these complications
tend to be disastrous for the afflicted
patient, as this case illustrates.

Secondary infections or superinfections of the skin are
the most common complication of varicella viral
infections and include abscess,
lymphadenitis, cellulitis, erysipelas, and
gangrene. Group A [beta]-emolytic
Streptococcus is the most commonly isolated bacterial
pathogen contributing to those
complications.[2] Gangrenous complications of
varicella occur at an estimated frequency of only
0.05% to 0.16% of cases.[3] The original
description of this dreaded complication is
credited to Stokes,[4] who in 1807 reported on an
eruptive disease of children. Hutchinson[5] coined
the term varicella gangrenosa, a more apt
description of this morbid and potentially fatal
complication of varicella
infection. Varicella gangrenosa likely represents one of
the many forms of necrotizing fasciitis
consequent to subcutaneous streptococcal infection
that can be seen in clinical practice. Such cases
of fasciitis are variously called
Fournier's gangrene, synergistic gangrene, and
Meleny's gangrene. Differentiation of this entity
from mucormycosis, which can produce rapidly
progressive gangrenous
changes in patients with diabetes, is important.
Antifungal therapy with intravenous amphotericin B
would be indicated if hyphal elements were
found on frozen or permanent sections of involved necrotic
tissue.[6]

Oral and intravenous acyclovir may reduce the incidence of
complications if given early in the course
of the disease in immunocompetent hosts, but
particularly in immunocompromised patients.[7]
Ideally, therapy with
acyclovir should begin as soon as possible after the first
vesicle appears at a dose of 800 mg orally
five times a day for 7 days in uncomplicated
varicella. If patients manifest complicated
varicella, intravenous acyclovir should be
administered at a dose of 10 mg/kg for 7 to 10
days. Our patient had multiple complications,
including cerebritis, encephalitis, brain abscess,
and facial/orbital
necrosis despite aggressive intravenous treatment
with acyclovir and broad spectrum antibiotic
medications before and after surgical treatment.

The early surgical management of our case with eyelid and
soft tissue debridement did not prevent
ongoing damage to the vital eye structures despite
subfascial debridement, which included some bony
structures. The secondary debridement
necessary in this case bespeaks of the potent
effects of subintegumentary infection with group A
[beta]-hemolytic streptococci complicating varicella viral
infections. Poor penetration of antimicrobial medication
into the areas of relative hypovascularity may
explain the poor response of this infection
to traditional treatment with intravenous
antibiotics, and therefore aggressive surgical debridement is mandated. Earlier diagnosis and surgical therapy with prompt debridement
may help reduce the extreme morbidity and
potential mortality incurred by varicella
gangrenosa. Our patient's severely compromised
neurologic status precluded safe skin grafting or periorbital reconstruction with free tissue transfers. The disfigurement induced by debridement of
gangrenous periorbital structures is probably best
remedied by manufacture of a prosthesis for aesthetic considerations.

Disclaimer: The Immunization Action Coalition (IAC) publishes
Unprotected People Reports for the purpose of making them available
for our readers' review. We have not verified the content of this
report.

This website is supported in part by a cooperative agreement from the National Center for Immunization and Respiratory Diseases (Grant No. 5U38IP000290) at the Centers for Disease Control and Prevention (CDC) in Atlanta, GA. The website content is the sole responsibility of IAC and does not necessarily represent the official views of CDC.